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Final clinical long case 38

Created: 26/10/2011
A 43-year-old male with Down’s syndrome, known to have an atrial septal defect, underwent an operation 3 months ago for cataract surgery to the right eye. This surgery failed. The patient has returned for a cataract operation in the left eye. He lives in sheltered accommodation and is needle-phobic.

On examination, he is plethoric and has a respiratory rate of 18/min, with a loud systolic murmur, best heard in the left sternum, second rib space.

On air, his PO2 is 10 mmHg, pH 7.4, PCO2 4.5 mmHg, [HCO3]- 22.9 mEq/L and base excess -1. His haemoglobin is 18 mg/dL. White cell count, platelets, urea and electrolytes and liver function tests are all normal.

1. You are asked to interpret the ECG.
2. You are shown a chest X-ray and are asked to identify the following three features:

i) upper lobe diversion of blood
ii) very prominent pulmonary hila
iii) rotated vertebral spines indicating a scoliosis.

3. Bearing in mind that patients with Down’s syndrome may have endocardial cushion defects (ostium primum and ostium secundum), would you be concerned about shunting in this case? What about pulmonary hypertension?

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